Circulation, Vol 89, 206-215, Copyright © 1994 by American Heart Association
A Leenhardt, E Glaser, M Burguera, M Nurnberg, P Maison-Blanche and P Coumel
BACKGROUND: Torsade de pointes is characterized not only by its particular
ECG pattern but by its context of congenital or acquired long QT syndrome
and the long coupling interval of the initial premature beat. METHODS AND
RESULTS: We observed 14 patients aged 34.6 +/- 10 years (mean +/- SD) with
no structural heart disease who presented with syncope related to a typical
ECG aspect of torsade de pointes. However, there was no evidence of long QT
syndrome, and the torsade had the unusual particularity of an extremely
short coupling interval of the first beat or of the isolated premature
beats (245 +/- 28 milliseconds). In 10 cases they deteriorated into
ventricular fibrillation. Four patients had a familial history of sudden
death. Only 2 patients had a tachyarrhythmia inducible by programmed
stimulation. At Holter recordings the heart rate variability was globally
and significantly depressed, the vagal limb of the autonomic nervous system
being predominantly affected. During a mean follow-up of 7 years there were
5 deaths (4 sudden). Nine patients are alive, 3 with implanted
defibrillators and 6 treated with verapamil alone. Unlike the other types
of antiarrhythmic agents including beta-blockers and amiodarone, verapamil
is in our experience the only drug apparently active on the arrhythmias;
however, it does not prevent sudden death. CONCLUSIONS: The short-coupled
variant of torsade de pointes should be identified because of their ECG
pattern and the risk of sudden death in young adults with no structural
heart disease.
ARTICLES
Short-coupled variant of torsade de pointes. A new electrocardiographic entity in the spectrum of idiopathic ventricular tachyarrhythmias
Cardiology Department, Lariboisiere University Hospital, Paris, France.
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